GUILLAIN BARRE SYNDROME

OTHER NAMES:
Landry’s Guiilain-Barre Syndrome
Landry’s Ascending Paralysis
Acute Idiopathic Polyneuritis
Acute Inflammatory Demyelinating
polyradiculoneuropathy
Acute Immune-mediated Polyneuritis
Acute Polyradiculitis
Acute Segmentally Demyelinating Polyradiculoneuropathy
Guillain-Barre-Strohl Syndrome

DEFINITION:
First described by Landry in 1860’s, derived its from a
description in 1916 by Georges Guillain and Jean Alexander
Barre, who noted that this was a paralyzing condition associated
with increased concentration of protein but not cells, in CSF.
The Guillain-Barre syndrome may last for weeks or months,
but recovery is the rule and is often complete, without
paralytic seqeulae or muscular atrophy. The components of this
syndrome are as follows: (1) Motor disturbances: progressive
weakness or paralysis beginning in the lower extremities and
ascending to involve the trunk upper extremities, and
ocassionally the cranial nerves. Deep reflexes are lost, but the
cutaneous reflexes are preserved. (2) Sensory disturbances,
including paresthesias and marked tenderness on pressure over
the affected muscles; objective sensibility is only slightly
disturbed. (3) Albuminocytologic dissociation: hyperalbuminosis
of the spinal fluid in the absence of an increase in the number
of cells is a constant diagnostic finding. GBS is a
demyelinative disease that affects the peripheral nerves.
• Is an acute infective or idiopathic polyneuritis
characterized by a slowly progressive, symmetric ascending
flaccid motor weakness sometimes confused with poliomyelitis.
(UP handout)
• Like myasthenia gravis, GBS may be an autoimmune disease
that occurs after surgery, a viral infection, or immunization
as a result of viral antigens triggering an autoimmune
reaction to myelin.
• Is a form of polyneuropathy characterized by slowly
progressive, symmetric ascending motor weakness sometimes
confused with poliomyelitis. (Shand’s Handbook)

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 • An inflammatory polyradiculoneuropathy is a demyelinating
disorder that produces an acute or subacute paralysis that
often begins in the lower extremity and then ascends.
(Halsted)
• Is a term given to a condition seen in patients with
viral encephalitis. It is characterized by the absence of
fever, pain or tenderness in the muscles, motor weakness and
the abolition of motor reflexes.
• Acute, rapidiy progressive, symmetric polyradiculo-
neuropathy that predominantly affects motor function.
(Clinical Symposia)
• An acquired, symmetrical polyradiculoneuropathy
characterized by an abrupt onset of ascending paralysis from
the upper extremities. It usually compromises respiration,
degulation, and cranial nerves.

EPIDEMIOLOGY:
GBS is the most frequently acquired demyelinating
neuropathy, with an incidence of 0.6-0.9 cases per 100,000.
This disease happens in both sexes, but with slight
predominance in males. It happens in all parts of the world and
in all seasons. It affects children and adults of all ages (8
months to 81 years old), but with attack rate highest in persons
5074 years old. It also increases in patients with Hodgkin’s
disease and by pregnancy or general surgery. It is one of the
most life-threatening disease of the PNS. Annual incidence is 1
to 2 cases per 100,000 persons in the United States and many px
spend weeks in the hospital I.C.U. before recovering normal
function. With some 2-5% dying of respiratory paralysis,
autonomic instability and the complications of tracheostomy.
* non-seasonal and non-epidemic
* slightly predominant in males
* affects all ages with peak at 20-50 y/o

ETIOLOGY:
The etiology is actually unknown.
A mild respiratory or gastrointestinal infection, like
those that are caused by Coxsackie, £cho and Cytomegaloviruses
preceed the neuritic symptoms by 1-3 weeks. Other preceeding
events include surgical procedures. Viral exanthems, and other
viral illness, mycoplasma infections and lymphomatous cases,
like Hogdkin’s disease. The administration of outmoded anti-

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rabies vaccines and A/New Jersey (Swine) influenza vaccine, were
associated with increased incidence in GBS. It can also occur
during the course of lymphoma or systemic lupus erythematosus.
Another suqgested etiology for GBS is nutritional
deficiency (Vit B and allergy).
It may also be caused by Herpes Infection, Cytomegalovirus,
which accounts for large number of cases.

PATHOPHYSIOLOGY:
The dominant histopathologic findings in ACUTE INFLAMMATION
OF PERIPHERAL NERVE, manifested as a perivenular and endoneurial
infiltration by lymphocytes, macrophages, and a few plasma
cells. The invading cells varying in number, from a sparse
seeding of perivenous space to large collection of mononuclear
cells disseminated throughout the entire (M). Although immune-
mediated segmented demyelination is considered the primary
lesion of GBS, damage to axons characterically is also present,
particularly at autopsy. Electron microscopy has identified an
early effect on myelin sheaths. The cytoplasmic processes of
macrophages penetrate the basement membrane of Shwann cells,
particularly the vicinity of the nodes of ranvier, and extend
between the myelin lamellae, stripping away the myelin sheath
from the axon. Ultimately, the remnants of the myelin sheath are
engulfed by the macrophages. REMYELINATION follows up before
DEMYELINATION and appears to be the same as that following
segmental demyelination from other causes.
Inflammatory foci at demyelination are widely distributed
throughout the entire PNS, although their intensity is so
variable that they may be difficult to identify in an individual
case. The most intense inflammatory reaction is often localized
in spinal et cranial motor roots, and the adjacent parts of both
cranial et spinal nerves. The posterior roots, the dorsal roots
ganglia. the autonomic ganglia. and the more distal parts of
nerves are also involved at lesser extent

SUMMARY OF PATHOPHYSIOLOGY:
(+) Endoneurial mononuclear infiltration-Focal
segmental demyelination or Patchy demyelination
of peripheral nerve.
Acute case- axons are spared
Severe/chronic case - axonal degeneration
occurs
Remyelination by Schwann cells and axonaal
growth in later stages

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PATTERNS OF INJURY:
Damage may occur to the axon, myelin sheath cell body,
supporting connective tissue or nutrient blood supply to nerves.
Three basic pathological processes occur:

1. Segmental Degeneration
There is scattered destruction of the myelin sheath,
which occurs without axonal damage. The primary lesion
affects the Schwann cells.

2. Wallerian Degeneration
Damage of the axon distally following its
interruption. The axon
disintegrates and the myelin breaks up into globules.
Regeneration of nerve is possible because the basement of
the Schwann cells survives and acts as a skeleton along
which the axon regrows.

3. Distal Axonal Degeneration

Damage to the cell body or to the axon will affect the
viability of the axon which will ‘die back’ from the
periphery. Loss of myelin sheath occurs as a secondary
event Recovery is slow here because the axon must
regenerate. When the cell body is destroyed, no regrowth
will occur.
Axonopathy is term used when only the axon is injured.
Myelopathy is used when only the myelin sheath is affected.

In contrast to the axons in the CNS, peripheral nerve

fibers have excellent capability to regenerate under proper
circumstances. The process of regeneration following axonal
degeneration may take from 2 months to more than a year.
Depending on the severity of the neuropathy and the length of
regeneration required. Whether regeneration takes place depends
upon the subsidence of the initial basis for neuropathy. This
could be removal form contact with a neurotoxic substance or
correction of an abnormal metabolic state. A deficit secondary
to demyelination may recover rapidiy since intact axons may
remyelinate in just a few weeks. In GBS, wherein demyelination
but no secondary axonal degeneration has occured, recovery to
normal strength from bedfastness and paralysis of arms and legs

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is as little as 3 to 4 weeks.

FUNCTIONAL DISABILITY
1. Excitability of axon may be increased with spontaneous or
excessive firing of an axon that results into
fasciculations.
2. The axon maybe enable to conduct an action potential-either
because of structural damage to axon or transient metabolic
change.
In severed axon, the distal portion will undergo Wallerian
degeneration, but the proximal portion has normal conduction.
3. The axon may conduct an impulse slowly or at low rate of
firing due to loss of myelin or narrowing & deformation of
axon. (axonal disruption)
4. Nodes of Ranvier may be impaired that results to less
formation of myelin sheaths.

The physiologic alteration seen with GBS are associated with the
ff. manifestation:
1. Diminution on loss of ability of peripheral nerves to
conduct impulse
2. Irritability in sensory fiber
3. Fasciculations due to ion threshold of activation of some
portion of the nerve.

SIGNS AND SYMPTOMS:

1. Distal paresthesia - due to the involvement of the
longest neurons which suffers first and undergo
degeneration.
2. Decresed pulmonary vital capacity - due to the
involvement of intercostal muscles and diaphragm.
3. Progressive skeletal muscle paralysis- due to the
complete conduction block that occurs proximally in
nerve roots.
4. Dysphagia - due to involvement of CN 9,10,12 and also
the bulbar.
5. Loss of DTR - this occurs if the conduction in the
afferent and efferent pathways are delayed or
dispersed due to demyelination of the peripheral
nerves. And if large number of la afferent or alpha
motoneurons have degenerated so that the afferent or
efferent responses are severely reduced in amplitude.
6. Peripheral facial weakness - due to CN 7 involvement

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 7. Distal glove and stocking anaesthesia - due to the
involvement of the longest neurons which is first to
be affected.
8. Cardiac Arrythinias - due to the affectations of the
autonomic functions.
9. Altered blood pressure - due to affectation of ANS
10.Decreased appreciation of vibration and
proprioceptive senses - due to axonal destruction.
11.Postural hypotension - due to involvement of the
preganglionic sympathetic fibers in the thoracic nerve
root.

ELECTROPHYSIOLOGY AND LABORATORY FINDINGS:

The CSF is under normal pressure and is acellular;
pleocytosis of 10 to 50 cells per cubic mm predominantly
lymphocytes are found. Several days after the onset of symptoms,
the protein level begins to rise, reaching a peak in 4 to 6
weeks. In the peripheral blood, there is moderate leukocytosis
and shift to immature forms early in the illness, but the blood
pressure soon returns to normal
Nerve conduction velocities are reduced in GBS, but studies
show it may be normal in the course. Distal sensory and motor
latencies are prolonged. Owing to the demyelination or nerve
roots, F-wave conduction is often showed. Severity of neurologic
abnormality correlates poorly with the degree of slowing of
nerve conduction velocities. Nerve conduction slowing may
persist for months or years after clinical recovery.
Increased protein level in CSF
cytoalbuminological dissociation
Normal number of cells EMG - best prognostic indicator

PROGNOSIS:
The majority of patients recover spontaneously and
completely. Death is unusual result of GBS but is generally due
to aspiration pneumonia, pulmonary embolism, intercurrent
infection or autoimmune dysfunction. Speed of recovery varies.
Often, it occurs within a few weeks or months but if axons have
denervated or degenerated, their regeneration may require 6 -l8
months or longer. In general, elderly patients’ recover more
slowly than younger ones, and are more likely to be left with
residual weakness, residual deficits or hyporeflexia
3 -5 % of patients do not survive the illness.

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COURSE & PROGNOSIS
1. Progression of paralysis reaches its max at 1 -3 weeks
2. Plateau period of 2-4 weeks after improvement from the
disease.
3. Recovery before the fourth week more often complete
recovery.
* prognosis is favorable in children
* axonopathy - indicated by needle EMG; have poorer prognosis
compared to demyelination.
DIAGNOSIS:

Acute Spinal cord lesions may be confused with this syndrome.

These lesions also cause rapidly progressive paralysis but
sensory examinations usually demonstrates
a spinal level The reflexes are usually hyperactive and
associated with positive Babinski sign. Spinal cord lesions also
typically cause early bladder and bowel dysfunction. Back pain
may be a feature in either disorder, but is common in SCL

Diptheric polyneuropathy can usually be distinguished by the

long latent period between the respiratory infection and onset
of neuritis, the frequency of paralysis of accommodation and the
relatively slow evolution of neuritic symptoms.

Acute anterior poliomyelitis can usually be distinguished by the

symmetry of the paralysis and by the presence of signs of
meningeal irritation, fever, and CSF pleocytosis early in the
course.

CRITERIA FOR DIAGNOSIS OF GBS:

1. Diffuse symmetric flaccid paralysis often associated with
bilateral facial paralysis.
2. Subjective sensory symptoms with objective sensory findings
are less remarkable than motor paralysis.
3. Complete remission within 6 months which occurs in almost
cases.
4. Increase in protein levels in the CSF beginning in the 1st
week of paralysis, with no or initially mild pleocytosis
(Albumin Cytologic dissociation)
5. Afebrile course or only slight increase in temperature during
the development of paralysis.
6. Normal WBC or lymphocytosis with little or no increase in
sedimentation rate.

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DIFFERENTIAL DIAGNOSIS:
1. ACUTE POLIOMYELITIS
- caused by human enterovirus through the oral-fecal route
affecting primarily the anterior horn cell resulting to
assymetrical limb involvement from proximal to distal and
hyporeflexia without alteration in sensory and autonomic
functions. CSF findings reveal upper cell count and lower
protein level NCV is normal

2. ACUTE SPINAL CORD LESIONS

- also causes rapidly progressive paralysis but sensory
examination usually demonstrates a spinal level
- Reflexes are usually hyperactive and associated with
positive Babinski sign.
- Typically cause early bladder and bowel dysfunction
3. BOTULISM
- electrophysiology test reveals normal NCV, facilitating
response to repetitive nerve stimulation

4. CHRONIC DEMYELINATING POLYNEURITIS

- symptoms progress from 6 mos. to 2 years. With frequent
recurrent attacks, Upper in CSF protein is less consistent
Also manifests with asymmetrical weakness and optic
neuritis with enlargement of the nerves due to Schwann
cell proliferation and collagen deposition after recurrent
segmental disease and remyelination.

5. DIPHTRERIC POLYNEUROPATHY
- can usually be distinguished by long latent period
between the respiratory infection and onset of neuritis,
the frequency of paralysis of accommodation and relatively
slow evolution of neuritic symptoms.

6. HYPOPHOSPHATEMIA INDUCED NEURAL DYSFUNCTION

- history of toxin exposure or in thallium or arsenic
intoxication
- subsequent development of alopecia

7. MILLER FISHER SYNDROME

- characterized by acute external ophthalmoplegia, ataxia.
areflexia with or without limb weaakness. This follows an
upper respiratory tract infection predominantly affecting
the males. Recovery is almost always complete.

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8. PERPHYRIC NEUROPATHY
- history or recurrent, mental symptoms and onset after
exposure to barbiturates or other drugs
- CSF proteins is not increased

9. POST-DIPHTHERIA POLYNEUROPATHY (PDP)

- with history of diphtheria

10. TICK PARALYSIS

- excluded by careful examination of the scalp.

11. TRANSMYELITIS
- it is a spinal cord affectation with marked sensory
deficit Autonomic symptom is usually earlier and not
associated with temepature.

12. MULTIPLE SCLEROSIS

- it can be unilateral or bilateraL Incontinence is usually
present but not
associated with temperature.

13. AIDS
- it develops relatively rapidly progressive demyelinating
polyneuropathy and HIV has been isolated from peripheral
nerve and CSF for some patient

GBS POLIOMYELITIS
ETIOLOGY Unknown polio virus
DISTIBUTION diffuse, generalized patchy flaccidity

symmetrical flaccidity

COURSE distal to proximal proximal to distal

STRUCTURES AFFECTED axon and myelin sheath anterior horn cells

SENSORY (+) affectation (-) affectation

paresthesia intact sensation

IN ASSOCIATION WITH weakness progresses days weakness is non

TEMPERATURE up to weeks with fever progressive

DTR’s Absent initially

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hyperreflexive

AUTONOMIC RESPONSE incontinence - rar none

ATROPHY Absent present

FUNCTIONS INVOLVE motor, sensory and motor only

autonomic

CSF FINDINGS Normal glucose normal glucose

(N)/low cell count upper cell count
upper CSF protein (N)/lower CSF
protein

NCV Slow normal

PROGNOSIS Good poor to fair

CLINICAL PHASE OF GUILLAIN-BARRR SYNPROME

Phase 1 - Tingling of hand and feet

Phase 2 - Difficulty in arising from chairs
Phase 3 - Areflexia, weakness, distal sensory loss
Phase 4 - Respiratory monitoring
Phase 5 - Mechanical ventilation
Phase 6 - Recovery stage, full activity

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